Document Detail

Familial syringomyelia: case report and review of the literature.
MedLine Citation:
PMID:  7482254     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Syringomyelia is an uncommon disease of the spinal cord, occurring sporadically. However, rare familial cases with autosomal dominant or recessive inheritance patterns are reported and their incidence quoted as approximately 2%. Only one previous report originated from the United States. METHODS: We present a brother and sister with syringomyelia and associated Chiari type I malformation; both patients responded to surgical treatment. We review the world literature and briefly discuss pathogenetic theories of syringomyelia as well as the relevance of the histocompatibility leukocyte antigen profile. RESULTS: Both genetic and environmental factors appear to be involved in familial syringomyelia. CONCLUSION: We recommend that close relatives of patients affected with familial syringomyelia undergo routine neurologic and radiologic surveys.
A Zakeri; F E Glasauer; J G Egnatchik
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Surgical neurology     Volume:  44     ISSN:  0090-3019     ISO Abbreviation:  Surg Neurol     Publication Date:  1995 Jul 
Date Detail:
Created Date:  1995-12-15     Completed Date:  1995-12-15     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  0367070     Medline TA:  Surg Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  48-53     Citation Subset:  IM    
Department of Neurosurgery, Millard Fillmore Hospital, Erie County Medical Center, Buffalo, New York, USA.
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MeSH Terms
Arnold-Chiari Malformation / complications
HLA Antigens / blood
Scoliosis / complications
Syringomyelia / complications,  genetics*,  immunology
Reg. No./Substance:
0/HLA Antigens

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